Dyskeratosis congenita with pancytopenia |
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| Authors: | HJ Dodd S DEVEREUX† I SARKANY |
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| Institution: | Department of Dermatology, Royal Free Hospital, Pond Street, Hampstead, London NW3 2QG;Department of Haematology, Royal Free Hospital, Pond Street, Hampstead, London NW3 2QG |
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| Abstract: | Dyskeratosis congenita is a rare inherited disorder characterized by skin pigmentation, nail dystrophy, leukoplakia and a variable number of additional features. This paper describes a patient who developed a serious manifestation, pancytopenia, as well as obliterated lacrimal puncta, gingivitis and loss of dermatoglyphics. The tendency to early malignancy is discussed and management by bone marrow transplantation and oral retinoids is proposed. |
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